Successful treatment with cyclosporin A for myelodysplastic syndrome with erythroid hypoplasia associated with T-cell receptor gene rearrangements

Myelodysplastic syndrome (MDS) with erythroid hypoplasia, a rare form of MD S, has not yet been clearly defined. We report four patients with MDS with erythroid hypoplasia who received immunosuppressive therapy. All were elder ly, had severe transfusion-dependent anaemia, morphological evidence of mye lodysplasia. and a low percentage (3.2-13.6%) of erythroid precursors. Admi nistration of cyclosporin A (CsA) improved their anaemia; all transfusion-d ependent patients achieved transfusion-independence. An inverted CD4/8 rati o was seen in three patients who also demonstrated T-cell receptor (TCR)-be ta and -gamma gene rearrangements by Southern blotting and clonality by pol ymerase. chain reaction. Treatment with CsA can be an attractive alternativ e treatment for patients with MDS with erythroid hypoplasia, which may be a ssociated with a clonal abnormality in T cells.